Hospital Clínic and Pediatric Hospital Sant Joan de Déu ......Penetrance of the disease increases...
Transcript of Hospital Clínic and Pediatric Hospital Sant Joan de Déu ......Penetrance of the disease increases...
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Brugada Syndrome:30 years after and still learning
Josep Brugada, MD, PhD
Hospital Clínic and Pediatric Hospital Sant Joan de DéuUniversity of Barcelona
Spain
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History
• 1st patient identified in 1986• 1st abstract including 4 patients in 1991
(Brugada P, Brugada J. PACE)• 1st paper including 8 patients in 1992
(Brugada P, Brugada J. JACC; 20, 1391-6)
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The ECG
- Prolonged PR
- RBBB
- ST segment ↑
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Ventricular arrhythmias
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Definition
The Brugada syndrome is a clinical-electrocardiographic diagnosis based on the occurrence of syncopal or sudden death episodes in patients without demonstrable structural heart disease and a characteristic ECG pattern of apparent right bundle branch block and ST segment elevation in leads V1 to V3.
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Original articles including in the title: Brugada syndrome
Number of articles Number of citations
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Initial descriptionJ Am Coll Cardiol
1992 1997 1998 1999 2000 2001 2002 2003 2005
Clinical descriptionJ Cardiovasc Electrofisiol
Sudden deathCirculation
GeneticsNature
Temperature dependenceCirc Res
AjmalineCirculation
EPSJ Cardiovasc Electrofisiol
ICDJ Electrocardiol
AsymptomaticsJ Cardiovasc Electrofisiol
Long term follow-upCirculation
SUDS = BrugadaHum Mol Genet
Diagnostic criteriaCirculation, Eur Heart J
MutationsMol Gen Metabol
Risk stratificationCirculation
ECGCirculation
ManagementCirculation, Eur Heart J
MutationsCirculation
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Type 1“Coved”
Type 2 & 3“Saddle back”
NON DIAGNOSTIC
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Figure 5. Spontaneous changes on the electrocardiogram in a patient with Brugada syndrome. Note how the ST elevation changes. On February 1993 the electrocardiogram was completely normal (arrow).
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basal ajmaline isoprot basal ajmaline isoprot
proband brother
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Ajmaline test
Affected Non-affected
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Value of the ajmaline test
• Sensitivity 80%• Specificity 94%• + PV 93%• - PV 83 %
Penetrance of the disease increases from 32,7% to 78,6% with the use of ajmaline
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Major events (SD or VF) depending on gender
Mujeres
Varones
Log-rank<0.003
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Type of ECG at baseline
Males Females
p < 0.001
Males Females
ST-segment elevation:
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Major events (SD or VF) depending on previous symptoms
Asintomáticos
Síncope
Muerte súbita
Log-rank<0.0001
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I am 28 years old, male. I was a very healthy individual.
I was on my duty about 10 p.m. I was talking on the phone when suddenly had lost my consciousness and hit the ground.
Luckily, I was seen by my co-resident … He immediately hooked me to a cardiac monitor.When I got my consciousness, I could not remember
what happened.…Can you confirm the diagnosis and tell me the steps I
need to follow to protect my family.
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Cardiac arrest
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Major events (SD or VF) depending on inducibility
No inducible
Inducible
Log-rank<0.0001
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Programmed ventricular stimulation
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N=166
EPSN= 135 (81%)
No EPSN= 31 (19%)
EPS (-)N= 89 (66%)
EPS (+)N= 46 (34%)
3 (10%) ICD1 (3%) Loop recorder
44 (96%) ICD1 (2%) Loop recorder
0 Arrhythmic events
1 (3%) SCDAsymptomaticBasal Type 1 ECG
Giustetto C et al. Europace (2009) 11,507-513.
7 VF (15%) interrupted by the ICD
1 VF (3%)interrupted by the ICD
FOLLOW-UP: 30 ± 21 months
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Multivariate analysis
Variable HR 95% IC P
Male 2,13 1,2-3,9 0,01
Syncope 3,77 2,2-6,3 < 0,001
SD 5,28 3,3-8,4 < 0,001
Inducibility 2,73 1,5-4,9 0,001
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ECG AT
BASELINE
ECG WITH AJMALINE
- - - + - - - - - - - - + - - + + + - - + N/A - N/A
MUTATION ANALYSIS WT WT T/M T/M T/M T/M WT WT T/M T/M WT T/M T/M
AFFECTEDNON AFFECTEDDEAD
Family K005
WT: Wild typeT/M:
T1620M
Phenotypic-Genotypic correlation
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I II III IV
Extracellular
Domains
COOH
NH2
*****
* R1623Q**R1644H***T1645M
S1710L1786
*
T1620M
R15
12W E1784K
D1790GN13
25S
ΔKPQ
150
5-15
07
G14
06R
1795insDY1795H
A19
24T
A19
24T
D15
95N
ΔK15
00
*
R1232W
T130
4MIV
S22+
2T>C
L567
Q T632MIVS7+4insAA
R1432G1397*
R11
92Q
A73
5V
R36
7H
G29
8S
G51
4C
Brugada syndromeLong QT syndromeConduction Disease
Brugada /Conduction DiseaseBrugada / LQT3 / Conduction Disease
• Identification of first gene in 19981:SCN5A (α-subunit of sodium channel) on chrom. 3
GENETICS
1. Chen, Brugada et al. Nature 1998
To date, > 200 different mutations identified in SCN5A2.
Loss of function of sodium channelOnly identified in ≈ 18-30% of
cases
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MOLECULAR STUDIESLow incidence SCN5A mutations/BS→genetic heterogeneity
Calcium cardiac channelsPotassium cardiac channelsSodium cardiac channels
IV
COOH
▪↓ I Na+ ↓ ICa++ AND/OR ↑ Ito ↑ Other IK
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Role of additional variations in Brugada Syndrome
Exon 28Exon 9
Exon 28
Exon 28 Exon 9
RB6043 RB6061
RB6042 RB6044
RB6045
?
RB6046
Exon 9
Cordeiro, Brugada et al. Circulation, 2006
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Role of additional variations in Brugada Syndrome
RB6043 RB6061
RB6042 RB6044
RB6045
RB6046
Cordeiro, Brugada et al. Circulation, 2006
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• Brugada syndrome (BS) = Na+ CHANNELOPATHY
MOLECULAR STUDIES
Ca++
- 90 mV
0 mV
Na+
Ito(K)
K+
AP in Brugada Syndrome SCN5A Mutation
Loss of functionNa+ channel
↓Na+ voltage-dependent current (INa)↑ K+ transient outward current (Ito)
•Loss of dome/AP
•ECG changes
•VT/VF
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Ionic mechanisms responsible for the phenotype of the Brugada syndrome are temperature dependent. Circ Res. 1999.
39.5°C 36.4°C
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Appropriate ICD therapies
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CLINICAL RECOMMENDATION FOR BRUGADA SYNDROME
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• Ito blockers:– QUINIDINE1 (Ito blocker with anticholinergic effects):
• 25 BS patients with inducible VF.• 22/25 (88%) not inducible after treatment.• 19/19 with no arrhythmias in follow-up.• 36% side effects → drug discontinuation.
– Other: tedisamil, 4-aminopyridine.• Activators of ICa:
– Isoproterenol (for electrical storm)2.– Cilostazol3.– Orciprenaline (for electrical storm) 4
PHARMACOLOGICAL TREATMENT
1. Belhassen et al. Circ 20042. Tanaka et al. PACE 2001
3. Tsuchiya et al. JCE 20024. Kyriasis et al. Europace 2009
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Reduction in the median number of ICD shocks before and after Quinidine administration.
P<0.001
Total numberof shocks n= 203
Total numberof shocks n= 41
Anguera I. et al, JACC 2016
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The Brugada ECG Pattern Ablation
Circulation 2002
Incidental abolition of Brugada pattern during endocardial ablation of ventricular ectopy for VF prevention in the RVOT
Journal of Cardiovascular Electrophysiology Vol. 22, pp. 1290-1291, November 2011
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• 17 arterially perfused canine RV preparations
• Pinacidil (5 μM) and pilsicaine (5 μM) → BS model
• RFCA earliest activation site of PVCs in EPI or ENDO
(N= 9)
(N= 8)
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Morita H et al. Heart Rhythm, Vol 6, Nº 5, May 2009
RFCA:AP heterogeneity still presentDisconnection of areas with long (EPI1) and short AP (EPI2) eliminated arrhythmia
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Circulation 2011
The Brugada ECG Pattern Ablation
- 9 patients were treated -In 7/9 pts no VT/VF was inducible-In 5/9 pts BrS pattern disappeared immediately after the procedure
-In 3/9 pts BrS pattern disappears during FUP
-In 8/9 patients no VT/VF recurrences occurred
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Brugada J, Pappone C, et al. Circ Arrhythm Electrophysiol 2015
-14 patients were enrolled (ICD was implanted in all patients)-We target fragmented and delayed potentials and low voltage areas in basal condition and after flecainide test
-In 14/14 pts Brugada pattern disappeared immediately after the RFA. -In 14/14 pts pharmacological test (flecainide or ajmaline) after RFA resulted negative-In 14/14 pts VT/VF was not inducible after RFA (EP Test up to three extrastimuli untill refractoriness or to 200 msec)
-In 14/14 pts during FUP Brugada ECG pattern was not evident anymore in basal condition and after drug challenge
-In 14/14 pts during FUP no recorded VT/VF episodes
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23/3/16 Gia
Basal Flecainide
Duration MAP
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23/3/16 Tar
Basal Flecainide RF on Flecainide Final
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23/3/16 Tar
Start RF RF on RF on RF on RF on RF on Final
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23/3/16 Tar
Basal
Flecainide
200 250 300
200 250 300
Duration MAP
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23/3/16 Tar
Basal
Final post RF
VOLTAGE DURATION ACTIVATION
VOLTAGE DURATION ACTIVATION
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Electrograms characterizationDiscrete double and late component
Wide duration, high voltage and delayed component
Wide duration, low voltage and late component
Uni - distal
Bip - distal
Bip - prox
V1 ECG lead
V2 ECG lead
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Potential Duration Map (PDM)Electrograms with longer duration, low voltage and late or consistent and discrete component are shown in purple color.
>200 ms >250 ms >280 ms252 ms
216 ms 252 ms 290 ms
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Automatic Potential Duration AnnotationAutomatic detectionRed Calipers
Onset Offset
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Color-code Map Potential Duration Map (PDM)
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Electrograms characterizationDiscrete double and late component
Wide duration, high voltage and delayed component
Wide duration, low voltage and late component
Uni - distal
Bip - distal
Bip - prox
V1 ECG lead
V2 ECG lead
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23/3/16 Gia
proximal
distal
proximal
distal
proximal
distalproximal
distal
proximal
distal
proximal
distal
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Case 2 SEE and Ajmaline Test pre ablationBASELINE AJMALINE 1 mg/Kg
IIIIII
aVRaVLaVFV1 II ic
V2 II icV1 III ic
V2 III icV1 IV ic
V2 IV ic
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6-months Follow-up - Ajmaline TestBASELINE
I
II
III
aVR
aVL
aVF
V1 II ic
V2 II icV1 III ic
V2 III ic
V1 IV ic
V2 IV ic
AJMALINE 1 mg/Kg
Case 2
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Bangungot = Lai Tai = Pokkuri = Brugada syndrome
“In Thailand, the being to fear is the phi am or ‘widow ghost’ who comes to steal away the souls of young men. Some villages think that bangungot appears in form of a huge and fat female demon, that sits over men’s face suffocating them, causing agonal respiration and ultimately death .Some men defend themselves from phi am by wearing lipstick at night, so that the ghost mistakes them for women and leaves them alone.”
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The Brugada family
Ramon Pedro Georgia Josep